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Malignant Solitary Fibrous Tumor of the Pleura with Concurrent Thymoma: A Case Report

孤立性肋膜纖維瘤合併胸腺瘤:病例報告及文獻回顧

摘要


孤立性肋膜纖維瘤是一種相當罕見的疾病。在過去認為這種腫瘤是由肋膜生長出,但藉由免疫組織化學染色技術的進步,我們瞭解事實上孤立性肋膜纖維瘤是由肋膜下肺間葉所生長出之腫瘤。臨床上並沒有特定的症狀增加術前診斷的困難度。常常單靠影象學並不能區分孤立性肋膜纖維瘤及其他肋膜腫瘤或肺癌合併肋膜侵犯。大多數此類腫瘤的病程緩和,但若為惡性形式則其病程難以預估。另外此類腫瘤的行為與組織學形態常不一致。手術切除應該是較好的治療方式。若是為非梗狀之惡性孤立性肋膜纖維瘤,手術切除後之復發率較高。今天我們提出一個病例,腫瘤位於左下胸腔內,同時合併有一前縱膈腔腫瘤。這位病人接受了經由開胸手術同時切除胸腺瘤及肋膜纖維瘤。最後的病理診斷為惡性孤立性肋膜纖維瘤及胸腺瘤,WHO type B1。目前術後七個月追蹤中且兩種疾病皆無復發現象。同時我們也進行一些文獻回顧。

並列摘要


Solitary fibrous tumors of the pleura (SFTP) are extremely rare. In the past, the origin of these tumors was thought to be mesothelial cells. With the development of immunohistochemical stain, we have found that SFTPs are subpleural mesenchymal tumors. Clinical preoperative diagnosis of the disease is difficult due to the absence of specific symptoms. Usually, SFTPs cannot be distinguished from other pleural tumors or lung tumors with pleural involvement, based on the image studies. Although the majority of these tumors have a benign course, the malignant form still remains enigmatic. The behavior of these tumors is often unpredictable and not correlated with the histologic findings. Surgical resection is a better treatment modality. The risk of recurrence is high after resection of a malignant sessile SFTP. Herein, we present the case of a mass in the left lower thorax with a concurrent anterior mediastinal tumor, which were simultaneously treated by excision of the mass in the pleura with wedge resection of the left lower lobe of the lung, and removal of the mediastinal tumor via an ipsilateral thoracotomy approach. The final pathologic diagnosis of the tumor was malignant SFTP and minimally invasive thymoma, WHO type B1. There was no evidence of recurrence of either disease in the 7-month follow-up after surgery. The related literature is also reviewed.

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